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International Journal of Dermatology Feb 2023
Topics: Pregnancy; Female; Humans; Pemphigoid Gestationis; Skin Pigmentation; Skin; Pregnancy Complications
PubMed: 35064677
DOI: 10.1111/ijd.16048 -
Experimental and Therapeutic Medicine Jan 2022Pemphigoid gestationis is considered to be a rare pregnancy exclusive bullous disease, which modifies the course of the pregnancy, with difficulties in the management of...
Pemphigoid gestationis is considered to be a rare pregnancy exclusive bullous disease, which modifies the course of the pregnancy, with difficulties in the management of the pruritus and skin lesions as well as a possible change in the neonatal outcome. Differential diagnosis of skin lesions and pruritus in pregnancy is challenging, and complementary investigations such as skin biopsy or laboratory tests are indispensable. The correct diagnosis and proper treatment could change the natural course of a pregnancy at risk and could improve maternal and fetal morbidity. We present the case of a patient with pemfigoid gestationis with the aim to highlight: i) the management of this pregnancy-associated skin disorder which transfers this pregnancy into a category of high obstetrical risk pregnancy; ii) the particularities of the course of the pregnancy; and iii) the importance in the differential diagnosis of pregnancy dermatoses. The particularity of this case of pemphigoid gestationis was the acute fetal distress in the absence of intrauterine growth restriction that is frequently found in this pathology, and the management of a rare pregnancy skin condition that currently has no standard treatment.
PubMed: 34815775
DOI: 10.3892/etm.2021.10945 -
Frontiers in Pharmacology 2020Pregnancy may induce the onset or exacerbation of autoimmune bullous diseases such as pemphigus or pemphigoid gestationis. A shift toward T helper (Th) 2 immune response... (Review)
Review
Pregnancy may induce the onset or exacerbation of autoimmune bullous diseases such as pemphigus or pemphigoid gestationis. A shift toward T helper (Th) 2 immune response and the influence of hormonal changes have been evoked as possible triggering factors. Therapeutic management of this setting of patients may represent a challenge, mainly due to safety concerns of some immunosuppressive drugs during pregnancy and lactation. In this narrative review, we provided a comprehensive overview of the therapeutic management of autoimmune bullous diseases in pregnant and breastfeeding women, focusing on pemphigus and pemphigoid gestationis.
PubMed: 33117178
DOI: 10.3389/fphar.2020.583354 -
Biomedical Papers of the Medical... Mar 2023Pemphigoid gestationis (PG) is a rare skin disease of pregnancy. Given its incidence in pregnant women, physicians and especially obstetricians may not encounter this...
BACKGROUND AND AIM
Pemphigoid gestationis (PG) is a rare skin disease of pregnancy. Given its incidence in pregnant women, physicians and especially obstetricians may not encounter this diagnosis in their entire career. We find this to be a major problem and there is an obligation to report it in as much detail as possible along with recommended treatments with proven efficacy.
CASE REPORT
We describe the case of a 27 year old patient who was referred to the dermatology department with severe dissemination of blisters in the 9th week of pregnancy. She was diagnosed with pemphigoid gestationis in her first pregnancy. High doses of corticosteroids were initiated but due to inadequate effect cyclosporine was added. The pregnancy was complicated with gestational diabetes. The patient gave birth in her 33rd week by caesarian section due to premature rupture of the membrane. Vesicles were seen on the newborn immediately after birth which diminished spontaneously over 2 weeks. Blisters were still seen on the patient 1 month after labor even with the combination of systemic corticosteroids with cyclosporine.
CONCLUSION
PG is a rare dermatosis of pregnancy. The course of the disease can be severe, necessitating systemic therapy. As described in this patient, systemic corticosteroids may not be sufficient and adding another immunosuppressive treatment may be needed. If pemphigoid gestationis has occurred during a previous pregnancy it is advised to reconsider another pregnancy.
PubMed: 36919665
DOI: 10.5507/bp.2023.010 -
Journal of Obstetrics and Gynaecology... Dec 2020Pemphigoid gestationis is a rare subepidermal bullous dermatosis of pregnancy, caused by the interaction of IgG1 auto-antibodies with 180 kD BP Antigen 2. This disease...
Pemphigoid gestationis is a rare subepidermal bullous dermatosis of pregnancy, caused by the interaction of IgG1 auto-antibodies with 180 kD BP Antigen 2. This disease can lead to preterm delivery, but the neonate is affected in only 10% cases. The treatment of choice in pemphigoid gestationis is systemic corticosteroids.
PubMed: 33417630
DOI: 10.1007/s13224-019-01302-7 -
The Australasian Journal of Dermatology Nov 2021The differentiation between the pemphigoid diseases is essential for treatment and prognosis. In Turkey, data on the incidence of these diseases are insufficient. Our...
BACKGROUND
The differentiation between the pemphigoid diseases is essential for treatment and prognosis. In Turkey, data on the incidence of these diseases are insufficient. Our aim in this study is to determine the incidence, demographics and clinical characteristics associated with diseases of the pemphigoid group.
METHODS
We prospectively analysed 295 patients with pemphigoid who visited dermatology clinics of tertiary referral hospitals in 12 different regions of Turkey within a year. The diagnosis was based on clinical, histopathological, direct immunofluorescence (DIF) and serological (multivariant enzyme-linked immunosorbent assay [ELISA], indirect immunofluorescence and mosaic-based BIOCHIP) examinations. Clinical and demographic findings, aetiological factors and concomitant diseases observed in the patients were recorded.
RESULTS
A total of 295 (female/male ratio: 1.7/1) patients with pemphigoid were diagnosed in 1-year period. The overall incidence rate of pemphigoid diseases was found to be 3.55 cases per million-years. The ratio of pemphigoid group diseases to pemphigus group diseases was 1.6. The most common pemphigoid type was bullous pemphigoid (BP, 93.2%). The others were epidermolysis bullosa acquisita (3.1%), pemphigoid gestationis (2.4%), linear IgA disease (1%) and mucous membrane pemphigoid (0.3%). The most common (26.8%) possible trigger of the bullous pemphigoid was gliptin derivative drugs. The most common concomitant diseases with pemphigoid were cardiovascular (27.8%) and neurological diseases (23.7%).
CONCLUSIONS
This study showed that the increased frequency of bullous pemphigoid reversed the pemphigoid/pemphigus ratio in Turkey. Further studies are warranted regarding the reasons for this increase.
Topics: Adolescent; Adult; Age Distribution; Aged; Aged, 80 and over; Child; Child, Preschool; Female; Humans; Incidence; Male; Middle Aged; Pemphigoid, Bullous; Pemphigus; Prospective Studies; Sex Distribution; Turkey; Young Adult
PubMed: 34642934
DOI: 10.1111/ajd.13731 -
JAAD Case Reports Mar 2023
PubMed: 36915856
DOI: 10.1016/j.jdcr.2023.01.026 -
Cureus Jan 2020Bullous pemphigoid is an autoimmune blistering disorder that typically presents in elderly patients as pruritic tense subepidermal blisters on the lower trunk, axilla,...
Bullous pemphigoid is an autoimmune blistering disorder that typically presents in elderly patients as pruritic tense subepidermal blisters on the lower trunk, axilla, and groin. It is caused by circulating and tissue-bound autoantibodies directed against bullous pemphigoid antigen 1 or bullous pemphigoid antigen 2 or both. Dyshidrosiform bullous pemphigoid is a rare variant of bullous pemphigoid, and it usually presents as itchy, potentially hemorrhagic, or purpuric blisters on the palms and/or soles of elderly individuals; subsequently, typical bullous lesions of bullous pemphigoid appear on other body sites. In our study, we report the features of two men with dyshidrosiform bullous pemphigoid and review the characteristics of individuals with this rare subtype of bullous pemphigoid. Including the men whose condition is described in this paper, at least 72 patients with dyshidrosiform bullous pemphigoid have been reported so far. However, complete features of the condition have not been described for all of the individuals. Based on the cases reported so far, the condition was slightly more common in women and the onset of the disease, for most of the patients, occurred between the ages of 61 and 94 years. The patients usually presented with blisters on both their palms and soles (66%) or just their soles (31%); 77% of the patients had progression of bullous pemphigoid to other areas of their body. Whether hemorrhagic blisters or purpuric lesions are associated with dyshidrosiform bullous pemphigoid remains to be determined; these features were present in 91% of the 22 patients who were described in the case reports yet were only observed in 5% of the individuals from a single larger series of 20 patients. The mainstay of therapy for dyshidrosiform bullous pemphigoid is systemic corticosteroids, with or without topical corticosteroids, and/or systemic dapsone or immunosuppressants; nearly all of the patients showed improvement after the treatment was initiated. Similar to individuals with bullous pemphigoid, at least nine of the dyshidrosiform bullous pemphigoid patients, including both patients in this report, had either a neurologic condition (seven patients) or both a neurologic condition and a psychiatric disorder (two patients). Usually, an autoimmune bullous disease, particularly dyshidrosiform bullous pemphigoid, is not initially considered in patients who present with blisters restricted to the palms and/or soles. Indeed, the lesion morphology of dyshidrosiform bullous pemphigoid mimics several other conditions that are characterized by blisters on the hands and feet, such as allergic and irritant contact dermatitis, chronic bullous disease of childhood, cutaneous T-cell lymphoma, dermatophyte infection, dyshidrosis or pompholyx, epidermolysis bullosa acquisita, erythema multiforme, herpes gestationis, lichen planus, linear IgA disease, scabies, and systemic contact dermatitis. In conclusion, the possibility of dyshidrosiform bullous pemphigoid should be considered in elderly individuals who present with the new onset of palmar and/or plantar blisters that are either recurrent or recalcitrant to therapy or would subsequently also appear on other areas of the body.
PubMed: 32064205
DOI: 10.7759/cureus.6630 -
Journal of the American Academy of... Jul 2023Pemphigoid gestationis (PG) and polymorphic eruption of pregnancy (PEP) may be similar morphologically but confer different maternal and fetal risks. Direct...
BACKGROUND
Pemphigoid gestationis (PG) and polymorphic eruption of pregnancy (PEP) may be similar morphologically but confer different maternal and fetal risks. Direct immunofluorescence is the gold standard test used to differentiate between the 2 diagnoses but is not always available.
OBJECTIVE
To develop and validate a clinical scoring system to differentiate PG from PEP.
METHODS
After developing a scoring system based on differentiating clinical factors reported in existing literature, we tested its diagnostic accuracy in a retrospective international multicenter validation study in collaboration with the European Academy of Dermatology and Venereology's Skin Diseases in Pregnancy Taskforce.
RESULTS
Nineteen pregnancies (16 patients) affected by PG and 39 pregnancies (39 patients) affected by PEP met inclusion criteria. PG had a mean score of 4.6 (SD, 2.5) and PEP had a mean score of -0.3 (SD, 2.0). The area under the curve was 0.93 (95% CI, 0.86-1.00). Univariate analysis revealed that almost all criteria used in the scoring system were significantly different between the groups (P < .05), except for skip pregnancy and multiple gestations, which were then removed from the final scoring system.
LIMITATIONS
Small retrospective study.
CONCLUSION
The Pregnancy Dermatoses Clinical Scoring System may be useful to differentiate PG from PEP in resource-limited settings.
Topics: Female; Pregnancy; Humans; Pemphigoid Gestationis; Retrospective Studies; Pruritus; Pregnancy Complications; Exanthema
PubMed: 36739091
DOI: 10.1016/j.jaad.2023.01.027 -
World Journal of Clinical Cases Dec 2021Pemphigoid gestationis (PG) is a rare autoimmune blistering disease that usually presents in the second or third trimester, with an incidence of 1 per 50000 pregnancies....
BACKGROUND
Pemphigoid gestationis (PG) is a rare autoimmune blistering disease that usually presents in the second or third trimester, with an incidence of 1 per 50000 pregnancies. PG tends to recur with an earlier onset and a more severe course in subsequent pregnancies. Skin biopsy markers can be confirmed by direct immunofluorescence staining.
CASE SUMMARY
Our patient was diagnosed with PG at 8 mo of gestation with fresh bullous lesion marks on the abdomen and limbs. Termination of the pregnancy was performed by cesarean section at 37 + 4 wk of gestation. The patient delivered an infant weighing 3620 gm. The infant had urticaria-like and vesicular skin lesions and was diagnosed with PG. The patient was discharged on prednisolone and in a satisfactory condition. The infant was discharged after anti-inflammatory therapy for one week.
CONCLUSION
PG is a rarely reported disease, and 10% of newborns develop mild clinical symptoms consisting of urticaria-like or vesicular skin lesions. We intend to remind clinicians to consider this condition when a patient presents with such lesions so that treatment can be started early and neonatal morbidity can be taken into account.
PubMed: 35004996
DOI: 10.12998/wjcc.v9.i34.10645